Sudden death due to primary diffuse leptomeningeal gliomatosis

Tumors of the central nervous system are an unusual cause of sudden death. This report describes the sudden death of a presumed healthy 28-year-old wo man from primary diffuse leptomeningeal gliomatosis. She presented to an em ergency room with headache and vomiting, subsequently became unresponsive a nd was pronounced dead 14 h later. Autopsy revealed a diffuse extensive inf iltrate of well-differentiated astrocytoma in the leptomeninges of the brai n and spinal cord without an underlying parenchymal tumor. Primary diffuse leptomeningeal gliomatosis is a rare tumor that arises within the leptomeni nges from small neuroglial heterotopic rests that undergo neoplastic transf ormation. Grossly, this tumor can mimic leptomeningeal carcinomatosis, pach ymeningitis, tuberculosis, sarcoidosis, and fungal infections. However, the histologic features of primary diffuse leptomeningeal gliomatosis should a llow it to be readily distinguished from grossly similar conditions. The me chanism of death in this case is most likely tumor obstruction of cerebrosp inal fluid outflow resulting in the usual complications seen with increased intracranial pressure. Although this tumor is aggressive and is associated with a rapidly progressive fatal course, it has not been previously associ ated with sudden death.